Oesophageal atresia and tracheo-oesophageal fistula.

The operative management of oesophageal atresia is reviewed. The operative technique and the approach to management, based on antomical classification of oesophageal atresia and classification into 3 different risk groups, are stressed. The embryology, aetiology, clinical features, symptoms and signs and radiological diagnosis of this condition are also briefly discussed.     

Current surgical management of oesophageal atresia and/or tracheo-oesophageal fistula

During the last 40 years, 579 babies with oesophageal atresia and/or tracheo-oesophageal fistula have been admitted to the Royal Children's Hospital (RCH), Melbourne. There are 393 survivors. Advances in the surgical management have contributed to lowering the morbidity and mortality rates. This paper describes current surgical management of each anatomical variant at RCH; specific reference is made to the problems of the long gap oesophageal atresia and prematurity.     

Intraoperative fibreoptic bronchoscopy during neonatal tracheo-oesophageal fistula ligation and oesophageal atresia repair.

Maintenance of adequate ventilation under anaesthesia can be difficult during identification and ligation of congenital tracheo-oesophageal fistula with repair of oesophageal atresia. Anaesthesia may also be complicated by problems associated with prematurity, pre-existing aspiration pneumonitis, and difficulty positioning the endotracheal tube to prevent inflation of the stomach with increased risk of aspiration and diaphragmatic splinting. Even intubation of the fistula and gastric rupture may occur. Two neonatal cases are presented where use of a 2.2 mm neonatal bronchoscope passed through a 3.0 mm ID tracheal tube facilitated surgical identification of the fistula, diagnosis of fistula intubation and other airway problems intraoperatively.     

Tracheal stenosis or agenesis in association with tracheo-oesophageal fistula and oesophageal atresia

Two babies are described with oesophageal atresia, a tracheo-oesophageal fistula and severe subglottic tracheal stenosis. A third baby, who did not survive, had a tracheal agenesis associated with bronchi arising from the oesophagus. A review of the types of tracheal stenosis and agenesis associated with various forms of tracheo-oesophageal fistula is included.     

Oesophageal atresia and tracheo-oesophageal fistula

Oesophageal atresia and tracheo-oesophageal fistula is a congenital structural abnormality that affects 1:4500 live infants. It is due to failure of the primitive foregut tube to separate correctly into oesophagus and trachea. About 50% have associated abnormalities, of which the VACTERL (Vertebral, Anorectal, Cardiac, Tracheo-oEsophageal, Renal and Limb) association is the most common. Prematurity is common and all have some degree of tracheomalacia. Surgery of the common type can be performed through a fourth interspace thoracotomy or by thoracoscopy. It involves division of the distal tracheo-oesophageal fistula and anastomosing together the two ends of the oesophagus. The absence of a distal fistula reveals itself as a “gasless abdomen” on plain radiology, and usually indicates a long gap between the blind oesophageal ends: this sometimes necessitates an oesophageal replacement if extensive oesophageal mobilization fails to achieve an end-to-end anastomosis of the oesophagus. Potential post-operative problems include anastomotic leak, anastomotic stricture, recurrence of the fistula, gastro-oesophageal reflux, oesophageal dysmotility, and food impaction. Survival is determined mainly by coexisting congenital abnormalities. The long-term risk of oesophageal malignancy is yet to be established. Isolated tracheo-oesophageal fistula (“H fistula”) can occur without atresia, and often presents after feeding has commenced. It is divided through a cervical incision.     

Oesophageal atresia and tracheo-oesophageal fistula

Oesophageal atresia and tracheo-oesophageal fistula is a congenital structural abnormality that affects 1:4500 live infants. It is due to failure of the primitive foregut tube to separate correctly into the oesophagus and trachea. About 50% have associated abnormalities, of which the VACTERL (Vertebral, Anorectal, Cardiac, Tracheo-oEsophageal, Renal and Limb) association is the most common. Prematurity is common and all have some degree of tracheomalacia. Surgery of the common type can be performed through a fourth interspace thoracotomy or by thoracoscopy. It involves division of the distal tracheo-oesophageal fistula and anastomosing together the two ends of the oesophagus. The absence of a distal fistula reveals itself as a ‘gasless abdomen’ on plain radiology, and usually indicates a long gap between the blind oesophageal ends: this sometimes necessitates an oesophageal replacement if extensive oesophageal mobilization fails to achieve an end-to-end anastomosis of the oesophagus. Potential postoperative problems include anastomotic leak, anastomotic stricture, recurrence of the fistula, gastro-oesophageal reflux, oesophageal dysmotility, and food impaction. Survival is determined mainly by coexisting congenital abnormalities. The long-term risk of oesophageal malignancy is yet to be established. Isolated tracheo-oesophageal fistula (‘H fistula’) can occur without atresia, and often presents after feeding has commenced. It is divided through a cervical incision.     

Oesophageal atresia and tracheo-oesophageal fistula

Oesophageal atresia and tracheo-oesophageal fistula is a congenital structural abnormality that affects 1:4500 live infants. It is due to failure of the primitive foregut tube to separate correctly into oesophagus and trachea. About 50% have associated abnormalities, of which the VACTERL (Vertebral, Anorectal, Cardiac, Tracheo-oEsophageal, Renal and Limb) association is the most common. Prematurity is common and all have some degree of tracheomalacia. Surgery of the common type can be performed through a fourth interspace thoracotomy or by thoracoscopy. It involves division of the distal tracheo-oesophageal fistula and anastomosing together the two ends of the oesophagus. The absence of a distal fistula reveals itself as a ‘gasless abdomen’ on plain radiology, and usually indicates a long gap between the blind oesophageal ends: this sometimes necessitates an oesophageal replacement if extensive oesophageal mobilization fails to achieve an end-to-end anastomosis of the oesophagus. Potential post-operative problems include anastomotic leak, anastomotic stricture, recurrence of the fistula, gastro-oesophageal reflux, oesophageal dysmotility and food impaction. Survival is determined mainly by coexisting congenital abnormalities. The long-term risk of oesophageal malignancy is yet to be established. Isolated tracheo-oesophageal fistula (‘H fistula’) can occur without atresia, and often presents after feeding has commenced. It is divided through a cervical incision.     

Dorsoventral patterning in oesophageal atresia with tracheo-oesophageal fistula: Evidence from a new mouse model

Background/Purpose: The well-established Adriamycin rat model of oesophageal atresia (OA) and tracheo-oesophageal fistula (TOF) complements recently described mouse genetic models in which loss of function mutations in foregut patterning genes, such as Nkx2.1 (Ttf 1), lead to OA/TOF. The authors aimed to integrate the 2 systems by adapting the Adriamycin model to the mouse to study molecular aspects of tracheo-oesophageal development. Methods: Pregnant CBA/Ca mice were injected intraperitoneally with 4 mg/kg of Adriamycin on embryonic days 7.5 and 8.5. Embryos and fetuses of various gestational ages were subjected to morphologic or histologic examination. Sections were stained with H [amp ] E or processed for immunohistochemistry using an antibody specific for Nkx2.1. Results: Tracheo-oesophageal malformations were observed in 47% of Adriamycin-treated embryos. Early foregut development was similar in Adriamycin-exposed and control embryos but, by E11.5, many treated embryos had an undivided oesophago-trachea, which gave rise to the lung buds and a fistula to the stomach. The fistula originated from the dorsal aspect of the undivided tube and was negative for Nkx2.1, or showed only transient Nkx2.1 expression, compared to the strongly positive bronchi ventrally. Conclusions: The Adriamycin model of OA is adaptable to the mouse. In the absence of tracheo-oesophageal separation, the dorsal fistula retains its nonrespiratory commitment suggesting that dorsoventral patterning of foregut development is undisturbed by Adriamycin exposure.     

H-type congenital tracheo-oesophageal fistula associated with oesophageal stenosis: anatomical variant

The goal of this paper was to report a new variant of oesophageal atresia: an H-type congenital tracheo-oesophageal fistula associated with oesophageal segmental stenosis distal to the fistula. Although symptoms were present from birth, we did not differentiate the new anatomical variant preoperatively. The patient was treated by fistula ligation, segmental resection of the distal oesophagus and end-to-end anastomosis of the oesophagus by thoracoscopic surgery. Here we describe the clinical history and management of the newborn infant, together with diagnostic recommendations to prevent misdiagnosis in the management of this condition.     

Management of giant acquired tracheo-oesophageal fistula in a neonate using an oesophageal patch

A neonate who underwent arterial switch operation had his postoperative course complicated by the development of tracheo-oesophageal fistula. He underwent multiple reparative procedures, ultimately requiring the oesophagus as a patch to close the large defect in the trachea and subsequent interposition of descending colon to restore the continuity of the upper gastrointestinal tract.